Chronic inflammatory demyelinating polyneurophaty: assessment of the cognitive function and quality of life.

BACKGROUND: Studies were carried out with the objective of evaluating the quality of life (QoL) of patients affected by chronic inflammatory demyelinating polyradiculopathy (CIDP). However, the cognitive issue is still little addressed. OBJECTIVES: To assess the QoL and cognitive impairment of patients with CIDP and to analyze whether there is a correlation between these parameters. METHODS: Seven patients with CIDP and seven paired controls were subjected to: mini-mental state examination (MMSE); Montreal cognitive assessment (MoCA); digit symbol replacement/symbol copy test (DSST); fatigue severity scale (FSS); Beck depressive inventory-I (BDI-I), and a short-form of health survey (SF-36). RESULTS: The mean age of the participants was 50 years (71.4% male). The MMSE and MoCA had no statistical difference between the groups. Patients showed superior results in the memory domain in the MoCA (5 vs. 2, p = 0.013). In the DSST, we observed a tendency for patients to be slower. There was a strong negative correlation between fatigue levels and vitality domain (SF-36). There was no significant correlation between depression levels and QoL, and there was no correlation between depression and the results obtained in the cognitive tests. The patients presented higher levels of depression (15.28 vs. 3.42, p < 0.001). A total of 57% had severe fatigue, 28.8% self-reported pain, and 57.1% complained of cramps. CONCLUSION: There was no cognitive impairment in these patients. However, there was a tendency of slower processing speed. To better evaluate the alterations found, a study with a larger number of individuals would be necessary. Chronic inflammatory demyelinating polyradiculopathy affects the QoL of patients in different ways.

ANTECEDENTES: Estudos foram realizados com o objetivo de avaliar a qualidade de vida (QV) de pacientes acometidos pela polirradiculopatia desmielinizante inflamatória crônica (PDIC). No entanto, a questão cognitiva ainda é pouco abordada. OBJETIVOS: Avaliar a QV e o comprometimento cognitivo em pacientes com PDIC bem como se existe correlação entre esses parâmetros. MéTODOS: Sete pacientes com PDIC e sete controles pareados foram submetidos a: miniexame do estado mental (MEEM); avaliação cognitiva de Montreal (MoCA); teste de substituição de símbolo de dígito/cópia de símbolo (DSST); escala de gravidade da fadiga (FSS); Beck depressive inventory-I (BDI-I) e um short-form of health survey (SF-36). RESULTADOS: A média de idade dos pacientes foi de 50 anos (71,4% do sexo masculino). O MMSE e o MoCA não apresentaram diferença estatística entre os grupos. Os pacientes apresentaram resultados superiores no domínio memória do MoCA (5 vs. 2, p = 0,013). No DSST, observamos uma tendência de os pacientes serem mais lentos. Houve forte correlação negativa entre os níveis de fadiga e o domínio vitalidade (SF-36). Não houve correlação significativa entre níveis de depressão e QV. Não houve correlação entre depressão e os resultados obtidos nos testes cognitivos. Níveis elevados de depressão foram observados nos pacientes (15,28 vs. 3,42, p < 0,001). Um total de 57% apresentou fadiga intensa, 28,8% dor autorreferida, e 57,1% queixam-se de câimbras. CONCLUSãO: Não há comprometimento cognitivo nos pacientes estudados. Observamos somente uma tendência de lentificação na velocidade de processamento. Para melhor avaliar as alterações encontradas, será necessário estudo com um número maior de indivíduos. A PDIC afeta de diferentes formas o nível de QV de seus portadores.

A Diffuse Leptomeningeal Glioneural Tumor Case Producing Hydrocephalus and Polyradiculopathy

The present report describes the case of a male 17-year-old patient who progressively developed a hydrocephalus and polyradiculopathy due to involvement of central nervous system (CNS) by a diffuse leptomeningeal glioneuronal tumor (DLGNT). The tumor had partial remission in response to the treatment with radiotherapy plus procarbazine, lomustine, and vincristine (PCV) chemotherapy, and the patient had improvement in function and pain levels. The current knowledge about DLGNT, including its clinical manifestations, imaging findings, histological characteristics, and treatment are revised and discussed in the present paper.

Síndrome de Guillain-Barre. Reporte de un caso / Guillaim Barre Syndrome. Case Report

Antecedentes. El Síndrome de Guillian-Barré (SGB) es una polirradiculopatía aguda, frecuentemente grave, de evolución fulminante sin la asistencia médica oportuna, de origen autoinmunitario. Que es precedida de un antecedente de infección gastrointestinal o respiratoria comúnmente. Se caracteriza por parálisis motora ascendente arrelexica de evolución rápida, con disociación albuminocitológica en líquido cefalorraquídeo. Las principales complicaciones se desarrollan en el 60% de pacientes intubados. Caso Clínico: masculino de 42 años de edad con parestesias en miembros inferiores de cuatro días de evolución, posteriormente a paresias que asciende rápidamente a miembros superiores. Como antecedente enfermedad gastrointestinal aguda hace 4 semanas. A la exploración clínica presentaba tetraparesia, arrelexia, ausencia de relejo tusígeno, y parálisis bulbar con posterior intubación orotraqueal y apoyo ventilatorio. Se realizó punción lumbar con disociación albuminocitológica. Se dio manejo con inmunoglobulina intravenosa (IgIV) por 5 días, siendo la insuiciencia respiratoria, neumonía nosocomial, sepsis y disautónomias como complicaciones más frecuente. Conclusión: el SGB es una entidad de pronóstico grave, que requiere de medidas de soportes esenciales para evitar complicaciones que puedan comprometer la vida del paciente...(AU)

[An adolescent with lumbosacral instability due to the presence of a hydatid cyst: a case report]. / Adolescente con inestabilidad lumbosacra por presencia de quiste hidatídico: reporte de caso.

Hydatidosis, caused by Echinococcus granulosus, affects the spinal column in 0.5-2% of all cases, with the sacral column being a rare location typically observed only in adults. The case is reported of a 14-year-old boy from Juliaca (Puno, Peru) who presented with chronic lower back pain and cauda equina syndrome. He was initially attended to in Juliaca, and then transferred to the Hospital Edgardo Rebagliati Martins (Lima, Peru) with a diagnosis of sacral tumor. In magnetic resonance imaging, a multicystic tumor was seen with L5 and S1 lysis and instability. The surgical treatment was an L5- S2 laminectomy and extraction of multiple hydatid cysts followed by lumbopelvic stabilization with transpedicular screws. Post-operatively, he was prescribed albendazol (15 mg/kg/d) for six months. Hydatid cysts should be considered within the differential diagnosis when there are cystic lesions in the spinal column, particularly in endemic areas such as Peru.

Adolescente con inestabilidad lumbosacra por presencia de quiste hidatídico: reporte de caso / An adolescent with lumbosacral instability due to the presence of a hydatid cyst: a case report

RESUMEN La hidatidosis, causada por el Echinococcus granulosus, afecta a la columna vertebral en 0,5-2% del total de casos, siendo la columna sacra una ubicación rara, típica de personas en edad adulta. Se reporta el caso de un varón de 14 años oriundo de Juliaca (Puno, Perú) quien presentó lumbalgia crónica y síndrome de cauda equina. Atendido inicialmente en Juliaca, fue trasladado al Hospital Edgardo Rebagliati Martins (Lima, Perú) con el diagnóstico de tumoración sacra. En la resonancia magnética se mostró una tumoración multiquística con lisis del L5 y S1 e inestabilidad. El tratamiento quirúrgico fue una laminectomía L5-S2 y extracción de múltiples quistes de hidátide más estabilización lumbopélvica con tornillos transpediculares. En el posoperatorio se recetó albendazol (15 mg/kg/d) por el periodo de seis meses. Se debe considerar al quiste hidatídico dentro del diagnóstico diferencial cuando existan lesiones quísticas en la columna vertebral, sobre todo en regiones endémicas como el Perú.

ABSTRACT Hydatidosis, caused by Echinococcus granulosus, affects the spinal column in 0.5-2% of all cases, with the sacral column being a rare location typically observed only in adults. The case is reported of a 14-year-old boy from Juliaca (Puno, Peru) who presented with chronic lower back pain and cauda equina syndrome. He was initially attended to in Juliaca, and then transferred to the Hospital Edgardo Rebagliati Martins (Lima, Peru) with a diagnosis of sacral tumor. In magnetic resonance imaging, a multicystic tumor was seen with L5 and S1 lysis and instability. The surgical treatment was an L5- S2 laminectomy and extraction of multiple hydatid cysts followed by lumbopelvic stabilization with transpedicular screws. Post-operatively, he was prescribed albendazol (15 mg/kg/d) for six months. Hydatid cysts should be considered within the differential diagnosis when there are cystic lesions in the spinal column, particularly in endemic areas such as Peru.

[Spinal extradural arachnoid cyst: A case report and review of literature]. / Quiste aracnoideo extradural espinal: reporte de un caso y revisión de la literatura.

BACKGROUND: Arachnoid cysts of spine are a very rare occurrence. The aetiology still remains unclear, but the most accepted explanation is the existence of areas of weakness in the spinal dura. Symptoms depend on the location in the spine. Magnetic resonance imaging is used for its diagnosis. Management depends of clinical presentation, and the surgery is reserved for patients with neurological impairment. CLINICAL CASE: A case is described of 67 year-old male with myelopathy and radiculopathy symptoms, both diagnosed simultaneously. The magnetic resonance imaging was used to diagnose a thoracolumbar extradural arachnoid cyst from T12-L2 and lumbar spinal canal stenosis. The patient was treated with a puncture procedure to empty the cyst and decompress the neural elements. There was a clinical improvement of myelopathy syndrome after puncture procedure. One month later, the patient underwent a minimally invasive surgical approach to decompress the neural elements in lumbar spine, achieving improvement of the radiculopathy syndrome and neurogenic claudication in both legs. CONCLUSION: There is currently no standard minimally invasive approach to surgically treat these cysts, but if the patient has mild symptoms, clinical observation is recommended.

Síndrome de Guillain Barré en pacientes pediátricos: diagnóstico y rehabiblitación. Reporte de Caso / Guillain-Barre Syndrome in Pediatric Patient: Diagnosis and Rehabilitation. Case Report

El Síndrome de Guillain-Barré (SGB) se deine como una polirradiculoneuropatía desmielinizante perifé - rica aguda. En América Latina es la principal causa de parálisis lácida en menores de 15 años con incidencia anual que oscila entre 0.91-1.1 casos por 100,000 habitantes. En Honduras un estudio de cohorte realizado durante 11 años en el Hospital Escuela Univer - sitario (HEU), estimó en la población pediátrica una incidencia de 1.37 casos por cada 100,000 habitantes. Caso clínico: Paciente femenina de 12 años de edad que fue diagnosticada con SGB variante neuropatía axonal motora aguda (AMAN), quien presentó falla ventilatoria y fue tratada con inmunoglobulina y esteroides. Permaneció 4 meses en unidad de cuidados intensivos pediátricos HEU donde se le realizaron exámenes para su diagnóstico y clasiicación, incluyendo citoquímica de líquido cefalorraquídeo que reporto disociación albuminocitológica a los 10 días de iniciado cuadro de debilidad muscular. Adicionalmente, electromiografía informó datos compatibles con SGB variante AMAN. Posteriormente fue referida al Servicio de Rehabilitación del Hospital de Especialidades San Felipe, donde inició terapia rehabilitadora por un lapso de 6 meses, presentando notable aumento de fuerza muscular y mejoría de independencia funcional. Conclusión: La terapia física y un buen aporte nutricional se han considerado factores clave para el manejo de los pacientes con SGB, sobre todo cuando la terapia se inicia de manera oportuna y multidisciplinaria...(AU)

Guillain-Barre syndrome as a differential diagnosis of low back pain syndrome / Síndrome de Guillain-Barré como diagnóstico diferencial de síndrome de dor lombar / El síndrome de Guillain-Barré como diagnóstico diferencial del síndrome de dolor lumbar

ABSTRACT Peripheral neuropathies are diseases of the lower motor neurons of the spinal cord and brainstem it can often mimic symptoms associated with the injuries of compressive radiculopathy. They are manifested by tingling, coldness, numbness, burning, pain, hypersensitivity, weakness, atrophy, postural hypotension, impotence, anhidrosis, and urinary incontinence. Therefore, it is important in the diagnostic strategy to keep in mind this type of pathology, especially when it comes to patients already in the 6th decade of life.

RESUMO As neuropatias periféricas são doenças dos neurônios motores inferiores da medula espinal e do tronco encefálico, cujos sintomas podem mimetizar, muitas vezes, lesões associadas à radiculopatia de origem compressiva. Manifestam-se por formigamento, frigidez, dormência, queimação, dor, hipersensibilidade, fraqueza, atrofias, hipotensão postural, impotência, anidrose e incontinência urinária. Portanto, é importante na estratégia de diagnóstico ter em mente esse tipo de patologia, principalmente quando se trata de doentes já na sexta década de vida.

RESUMEN Las neuropatías periféricas son trastornos de las neuronas motoras inferiores de la médula y del tallo encefálico, cuya sintomatología puede semejar muchas veces lesiones asociadas a una radiculopatía de origen compresiva. Se manifiestan por sensación de hormigueo, frialdad, adormecimiento, quemadura, dolor, hipersensibilidad, debilidad, atrofias, hipotensión arterial postural, impotencia, anhidrosis e incontinencia de esfínteres. Por lo tanto, es importante en la estrategia diagnóstica tener presente este tipo de patologías principalmente cuando se trata de pacientes que pasan la 6ª década de la vida.

Implicaciones anestésicas en paciente embarazada con Síndrome de Guillain Barré / Anesthetic implications in pregnant patient with Guillain-Barré Syndrome

Introducción: el Síndrome de Guillain Barré (SGB) es una enfermedad de baja incidencia en medicina y aún más raro durante la gestación, con una incidencia de 1,7/100.000 embarazos. La presentación GBS durante el embarazo se asocia con un aumento de la necesidad de asistencia respiratoria y aumento de la mortalidad materna. Métodos: describimos en este documento cómo fue el manejo perioperatorio de un caso de una mujer de 25 años de edad multípara que cursa un embarazo a término con SGB. Resultados: discusión del caso clínico. Conclusiones: no hay pautas establecidas para el manejo anestésico de una paciente embarazada con GBS.

Introduction: Guillain Barre Syndrome (GBS) is a rare occurrence in medicine, and possibly even rarer in pregnancies, with an incidence of 1.7/100.000 pregnancies. GBS occurrence in pregnancy is associated with an increased need for ventilatory support and increased maternal mortality. Methods: we describe herein how a case of a 25 year old multiparous at term gestation with GBS was managed in the perioperative period. Results: discussion of the case. Conclusions: there are no established guidelines for the anesthetic management of a pregnant GBS patient.

Estudo radiográfico da articulação lombossacra em cães das raças Pastor Alemão, Rottweiler e Doberman / Radiographic study of the lumbosacral joint in German Shepherd, Rottweiler and Doberman dogs

Este trabalho teve como objetivo avaliar a variação entre diferentes raças das medidas radiográficas da articulação lombossacra. Foram utilizados 20 cães da raça Pastor Alemão, 20 cães da raça Rottweiler e 20 da raça Doberman. A articulação lombossacra foi avaliada radiograficamente com os membros pélvicos em posição neutra (N), em ventroflexão (VF) e dorsoextensão (DE). Foram mensurados os ângulos entre os processos articulares de L7 e S1 (PA), entre as epífises distal de L7 e proximal de S1 (EPIF), o ângulo lombossacro (LS), a amplitude de movimento (ROM) desses ângulos, o ponto de interseção das retas que formam esses ângulos e a respectiva ROM. Os resultados mostraram que a altura do canal vertebral em L7 é menor no Pastor Alemão (9,5mm) e maior no Rottweiler (10,5mm). O EPIF-DE foi maior no Pastor Alemão (38,03º). O PA-N foi menor no Pastor Alemão (15,98º). O LS-VF foi menor no Doberman (170,01º), e não diferiu entre o Pastor Alemão (179,17º) e o Rottweiler (176,61º). Os cães Pastores Alemães demonstraram uma maior ROM dos ângulos EPIF e LS. Concluiu-se que cães da raça Pastor Alemão apresentam maior instabilidade dessa articulação em relação às outras duas raças estudadas.(AU)

The purpose of this work was to evaluate the racial difference of radiographic measurements of canine lumbosacral joints.20 German Shepherd, 20 Rottweiler and 20 Doberman dogs were used in the study. The lumbosacral joint was assessed radiographically with the hind limbs in neutral position (N), in ventral flexion (VF) and dorsal extension (DE). We measured the angles between the L7 and S1 articular processes (PA), between the distal L7 and S1 proximal epiphysis (EPIF), the lumbosacral angle (LS), range of motion (ROM) of these angles, the intersection point of the lines that form these angles and their ROM. The results show that the height of the spinal canal in L7 is lower in German Shepherds (9.5mm) than in Dobermans (10mm) and Rottweilers (10.5 mm). The EPIF-DE was higher in the German Shepherd (38.03°). The PA-N was lower in German Shepherd (15.98 °). The LS-VF was lower in the Doberman (170.01°), and LS did not differ statistically between German Shepherd (179.17°) and Rottweiler (176.61°). The ROM of the LS and EPIF angles were greater in the German Shepherd dogs. It was concluded that the German Shepherd dogs have radiographic measurements that lead to greater instability of the lumbosacral joint.(AU)

Differentiation of neonatal dorsal root ganglion-derived neural stem cells into oligodendrocytes after intrathecal transplantation into a cauda equina lesion model.

Cauda equina syndrome (CES) is characterized by varying patterns of low back pain, sciatica, lower extremity sensorimotor loss, and bowel and bladder dysfunction. The prognosis for complete recovery of CES is dependent on not only the time before surgical intervention with decompression but also the severity of the nerve damage. Delayed or severe nerve compression impairs the capability of nerve regeneration. Transplantation of neural stem cells (NSCs) may facilitate axon regeneration and functional recovery in a spectrum of neurological disorders. Our study shows that the NSCs derived from early postnatal dorsal root ganglion (DRG) are able to proliferate to form neurospheres and differentiate into O4(+) oligodendrocytes but not glial fibrillary acidic protein (GFAP(+)) astrocytes or ßIII-tubulin(+) neurons in vitro. After intrathecal transplantation into the lumbar spinal canal stenosis animal model, most of the GFP-expressing NSCs were induced to differentiate into oligodendrocytes in vivo. Although the recovery of sensorimotor function was not significantly improved in rats with transplantation therapy, our results implied that subarachnoid microinjection of NSCs may promote axon regeneration of DRG neurons in the cauda equina model after nerve injury.

Estudo epidemiológico da síndrome da cauda equina / Epidemiological study of cauda equina syndrome

Objetivo: O objetivo primário deste estudo é analisar as características e desfechos dos casos internados por síndrome da cauda equina (SCE) em nosso serviço. Secundariamente, este trabalho servirá de base para outros estudos comparativos visando um entendimento maior da doença e de sua epidemiologia. Métodos: estudo retrospectivo dos prontuários das internações por SCE no IOT-HCFMUSP no período de 2005 a 2011 com diagnósticos de SCE e bexiga neuropática. As seguintes variáveis foram analisadas: sexo, idade, etiologia da doença, nível topográfico da lesão, tempo de história da lesão até o diagnóstico, presença de bexiga neurogênica, tempo entre o diagnóstico da SCE e a cirurgia, reversão do déficit ou da bexiga neurogênica. Resultados: por tratar-se de uma doença rara, não conseguimos estabelecer correlações estatisticamente significativas entre as variáveis analisadas e os desfechos da doença. Porém, este estudo evidenciou deficiências do nosso sistema público de saúde quanto ao manejo desses pacientes. Conclusão: o trabalho mostra que apesar de bem definidas as bases para conduta da SCE, observamos um número maior de sequelas causadas pela patologia, do que visto na literatura. O atraso no diagnóstico e, a partir deste, do tratamento definitivo, foi a causa para o alto número de sequelas. Nível de evidência IV, Série de Casos.

Objective: The primary purpose of this study was to determinate the characteristics and outcomes of the patients admitted at our clinics diagnosed with cauda equina syndrome (CES). Secondarily, this study will serve as a basis for other comparative studies aiming the better understanding of this condition and its epidemiology. Methods: we conducted a retrospective study by reviewing the medical records of patients diagnosed with CES and neurogenic bladder between 2005 and 2011. The following variables were analyzed: gender, age, etiology, topographic level of the lesion, time between disease onset and diagnosis, presence of neurogenic bladder, time between diagnosis and surgery, neurological damage and neurogenic bladder persistence. Results: considering that CES is a rare condition, we were not able to establish statistic correlation between the analyzed variables and the outcomes of the disease. However, this study brought to light the inadequacy of our public health system in treating that kind of patient. Conclusion: The study shows that despite the well-defined basis for managing CES, we noted a greater number of patients with sequels caused by this condition, than it is seen in the literature. The delayed diagnosis and, consequently, delayed treatment, were the main causes for the results observed. Level of Evidence IV, Case Series.

Intradural lumbar disc herniation: report of five cases with literature review.

INTRODUCTION: Intradural lumbar disc herniations are uncommon presentations of a relatively frequent pathology, representing less than 1% of all lumbar disc hernias. They show specific features concerning their clinical diagnosis, with a higher incidence of cauda equina syndrome, and their surgical treatment requires a transdural approach. METHODS: In this article, we describe five cases of this pathology and review the literature as well as some considerations about the difficulties in the preoperative diagnostic issues and the surgical technique. CONCLUSION: We concluded that for intradural disc herniations the diagnosis is mainly intraoperative, and the surgical technique has some special aspects.

Síndrome de la cola de caballo secundario a hernia discal traumática / Horse-tail syndrome secondary to traumatic disc herniation

La cola de caballo representa la continuación caudal de la médula espinal. Esta porción del sistema nervioso es asiento de numerosas patologías. Se presenta un caso clínico que debutó de forma aguda con un síndrome de la cola de caballo, secundario a una hernia discal lumbar traumática. Se trata de un paciente masculino de 45 años de edad, que acude al servicio de urgencias, porque refiere síntomas que inician siete días antes de forma aguda y progresiva, luego de haber caído de una altura de tres metros. Al interrogarlo existía debilidad muscular del miembro inferior izquierdo y dificultad para caminar. El examen físico corroboró la disminución de la fuerza muscular y arreflexia Aquileo-plantar. Se realizó estudio de resonancia magnética, que demostró una hernia discal extruída en el interespacio L5-S1. El paciente se llevó al salón de urgencia y se realizó exeresis de la hernia. Egresó al séptimo día con discreta mejoría clínica. Se ha seguido en consulta externa durante un periodo de 8 meses, con muy poca recuperación del control esfinteriano, de la actividad motora y sensitiva(AU)

Horse-tail represents the caudal continuation of the spinal cord. This portion of the nervous system is the seat of numerous pathologies. We present a case report of acute debuted with a syndrome of the cauda equina, secondary to traumatic lumbar disc herniation. This is a male patient aged 45, who was admitted to the emergency room because he referred acute and progressive symptoms starting seven days before, after falling from three meters high. By questioning, we found muscle weakness in his left leg and difficulty at walking. The physical examination confirmed the decrease in muscle strength and Achilles-plantar areflexia. We performed MRI, which showed extruded disc herniation in L5-S1 interspace. The patient was taken to the emergency room and we performed the hernia excision. He was discharged at the seventh day with mild clinical improvement. We have continued the follow up as outpatient for a period of eight months. There is very little recovery of sphincter control, motor and sensory activity(AU)

Síndrome de la cola de caballo secundario a hernia discal traumática / Horse-tail syndrome secondary to traumatic disc herniation

La cola de caballo representa la continuación caudal de la médula espinal. Esta porción del sistema nervioso es asiento de numerosas patologías. Se presenta un caso clínico que debutó de forma aguda con un síndrome de la cola de caballo, secundario a una hernia discal lumbar traumática. Se trata de un paciente masculino de 45 años de edad, que acude al servicio de urgencias, porque refiere síntomas que inician siete días antes de forma aguda y progresiva, luego de haber caído de una altura de tres metros. Al interrogarlo existía debilidad muscular del miembro inferior izquierdo y dificultad para caminar. El examen físico corroboró la disminución de la fuerza muscular y arreflexia Aquileo-plantar. Se realizó estudio de resonancia magnética, que demostró una hernia discal extruída en el interespacio L5-S1. El paciente se llevó al salón de urgencia y se realizó exeresis de la hernia. Egresó al séptimo día con discreta mejoría clínica. Se ha seguido en consulta externa durante un periodo de 8 meses, con muy poca recuperación del control esfinteriano, de la actividad motora y sensitiva

Horse-tail represents the caudal continuation of the spinal cord. This portion of the nervous system is the seat of numerous pathologies. We present a case report of acute debuted with a syndrome of the cauda equina, secondary to traumatic lumbar disc herniation. This is a male patient aged 45, who was admitted to the emergency room because he referred acute and progressive symptoms starting seven days before, after falling from three meters high. By questioning, we found muscle weakness in his left leg and difficulty at walking. The physical examination confirmed the decrease in muscle strength and Achilles-plantar areflexia. We performed MRI, which showed extruded disc herniation in L5-S1 interspace. The patient was taken to the emergency room and we performed the hernia excision. He was discharged at the seventh day with mild clinical improvement. We have continued the follow up as outpatient for a period of eight months. There is very little recovery of sphincter control, motor and sensory activity

Paget's disease of bone: analysis of 134 cases from an island in Southern Brazil: another cluster of Paget's disease of bone in South America.

Paget's disease of bone (PDB) exhibits a marked geographic variation. In Brazil, the prevalence of PDB is unknown and only a few clinical data are available. The aim is to determine clinical, laboratory, imaging and response to treatment data in a large PDB case series in the city of Florianopolis, Brazil. We have performed a retrospective study based on charts reviews of all patients with PDB followed at the University Hospital of the Federal University of Santa Catarina and at five different private rheumatology outpatient offices in Florianopolis, between 1995 and 2009. One hundred and thirty-four patients with PDB were identified. Mean age at diagnosis was 63.2 ± 10.5 years, 67.2% were women, and 91.1% were Caucasian. Positive family history was reported in only 8.2%. Polyostotic disease was found in 75.0% of the cases, bone pain in 77.9%, and bone deformities in 15.9%. Higher levels of AP were significantly associated with polyostotic disease and skull involvement. Pelvic bones were the most frequently affected (53.7%). Complications included deafness in 8.2%, bone fractures in 3.0%, hydrocephalus in 2.2%, and cauda equina syndrome in 0.7% of the cases. Treatment with zoledronic acid achieved the best response with only 2.9% failing to respond adequately. According to literature data, PDB in South America seems to be characterized by an overall low prevalence, but with localized clusters with higher prevalence. The authors have described a cluster of PDB in Florianopolis, in Southern Brazil. Further properly designed studies are necessary to clarify the PDB epidemiology in South America.